CPN1


Description

The CPN1 (carboxypeptidase N subunit 1) is a protein-coding gene located on chromosome 10.

The CPN1 gene encodes the catalytic subunit of Carboxypeptidase N, a plasma metallo-protease essential for regulating peptides like kinins and anaphylatoxins. This enzyme is a tetramer with two identical regulatory subunits and two identical catalytic subunits. It cleaves basic amino acids from the C-terminal end of peptides and proteins, effectively controlling their activity. CPN1 deficiency, due to mutations in this gene, can lead to angioedema or chronic urticaria. In melanocytic cells, CPN1 gene expression may be regulated by MITF.

CPN1 plays a vital role in regulating inflammatory responses by inactivating potent vasoactive and inflammatory peptides like kinins and anaphylatoxins, which contain C-terminal Arg or Lys residues and are released into the bloodstream.

CPN1 is also known as CPN, SCPN.

Associated Diseases


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